Surgical, Hybrid, or Delayed Surgical Stage 1: Single-Ventricle Palliation Pathway Utilization and Outcome Trends

Pathways for single-ventricle palliation include traditional neonatal surgical stage 1 palliation (SP), hybrid stage 1 palliation (HP; pulmonary artery flow restriction with ductal stenting), and delayed surgical stage 1 palliation (DSP), preceded by pulmonary artery flow restriction and prostaglandins. Findings from studies aiming to determine the optimal pathway are conflicting. We aimed to describe current pathway utilization and outcomes.

Using the Pediatric Health Information System between January 2016 and August 2023, we identified 1872 patients who underwent single-ventricle palliation (1573 SP, 123 DSP, and 176 HP).

Prematurity or low birth weight (<2.5 kg) were most common for DSP (52.9%) compared with SP (15.3%) and HP (36.4%) (P<0.001). Comorbid conditions were most common for DSP. Case selection varied based on hospital volume; centers in the lowest volume quintile performed relatively more HP and DSP. In-hospital mortality and index hospitalization transplant were highest among HP (25.6%, 8.5%) compared with DSP (13.8%, 0.8%) and SP (11.3%, 0.9%) (P<0.001). Hospital and intensive care unit length of stay were highest for DSP (both P<0.001). Patients born prematurely or with low birth weight showed the highest in-hospital mortality for HP (35.9%) compared with DSP (16.9%) and SP (19.9%) (P=0.012); postprocedure length of stay was longest for DSP (P=0.026). The costs per day did not vary.

DSP was used for patients with a higher rate of prematurity, lower birth weight, and more noncardiac comorbid conditions. HP, however, was associated with higher in-hospital mortality and index hospitalization transplant. Further study to determine the potential benefit of DSP for high-risk patients is warranted.